LUMIZYME Dosage & Rx Info | Uses, Side Effects

Lumizyme Generic Name & Formulations

Legal Class

General Description

Alglucosidase alfa 50mg/vial; lyophilized pwd for IV infusion after reconstitution and dilution; preservative-free.

Pharmacological Class

Hydrolytic lysosomal glycogen-specific enzyme.

How Supplied

Single-use vial—1, 10

Manufacturer

Sanofi Genzyme Company

Generic Availability

Mechanism of Action

Alglucosidase alfa provides an exogenous source of acid α-glucosidase (GAA). After binding to mannose-6-phosphate receptors on the cell surface, it becomes internalized and transported into lysosomes, where it undergoes proteolytic cleavage that results in increased enzymatic activity. It then exerts enzymatic activity in cleaving glycogen.

Lumizyme Indications

Indications

Pompe disease (GAA deficiency).

Lumizyme Dosage and Administration

Adults and Children

Consider premedication with antihistamines, antipyretics, and/or corticosteroids. Give by IV infusion over 4hrs. 20mg/kg every 2 weeks. Initial infusion rate: max 1mg/kg/hr; may increase by 2mg/kg/hr every 30mins, if tolerated, to max 7mg/kg/hr.

Lumizyme Contraindications

Not Applicable

Lumizyme Boxed Warnings

Boxed Warning

Hypersensitivity reactions including anaphylaxis. Immune-mediated reactions. Risk of cardiorespiratory failure.

Lumizyme Warnings/Precautions

Warnings/Precautions

Risk of hypersensitivity reactions including anaphylaxis; discontinue immediately if develop and treat appropriately. Have appropriate medical support available and during rechallenge. Decrease infusion rate, temporarily stop infusion, and/or give antihistamines and/or antipyretics if infusion associated reactions develop. Monitor for systemic immune-mediated reactions; consider discontinuing and treat if occur. Obtain urinalysis periodically. Acute underlying respiratory illness or compromised cardiac and/or respiratory function: risk of acute cardiorespiratory failure. Cardiac hypertrophy (increased risk of cardiac arrhythmia when given general anesthesia for central venous catheter placement). Infants with cardiac dysfunction may require prolonged observation times. Risk of antibody development. Infantile-onset disease: perform cross-reactive immunologic material (CRIM) assessment early in the disease course. Monitor for IgG antibody formation every 3 months for 2 years, then annually. Labor & delivery. Pregnancy. Nursing mothers (consider interrupting breastfeeding, pumping and discarding breast milk during and for 24hrs after therapy).

Lumizyme Pharmacokinetics

Elimination

Half-life: 2.3 hours. Clearance: 25 mL/hr/kg.

Lumizyme Interactions